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Diffuse Intrinsic Pontine Glioma
Luke L. Linscott, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Diffuse intrinsic pontine glioma (DIPG)
              • Synonyms

                • Pontine glioma
              • Definitions

                • Imaging diagnosis that includes multiple histologic & molecular glial tumor subtypes
                • Glial tumor centered in pons (WHO grades II-IV)
                  • Expansile, often poorly marginated, typically lacks enhancement
                  • Glioblastoma shows focal enhancement & central necrosis

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              • Reporting Tips

                                                Selected References

                                                1. Pfaff E et al: Brainstem biopsy in pediatric diffuse intrinsic pontine glioma in the era of precision medicine: the INFORM study experience. Eur J Cancer. 114:27-35, 2019
                                                2. Harward S et al: T2-weighted images are superior to other MR image types for the determination of diffuse intrinsic pontine glioma intratumoral heterogeneity. Childs Nerv Syst. 34(3):449-55, 2018
                                                3. Hoffman LM et al: Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): a collaborative report from the International and European Society for Pediatric Oncology DIPG Registries. J Clin Oncol. 36(19):1963-72, 2018
                                                4. Johung TB et al: Diffuse intrinsic pontine glioma: new pathophysiological insights and emerging therapeutic targets. Curr Neuropharmacol. 15(1):88-97, 2017
                                                5. Mackay A et al: Integrated molecular meta-analysis of 1,000 pediatric high-grade and diffuse intrinsic pontine glioma. Cancer Cell. 32(4):520-37.e5, 2017
                                                6. Hoffman LM et al: Spatial genomic heterogeneity in diffuse intrinsic pontine and midline high-grade glioma: implications for diagnostic biopsy and targeted therapeutics. Acta Neuropathol Commun. 4:1, 2016
                                                7. Poussaint TY et al: Apparent diffusion coefficient histogram metrics correlate with survival in diffuse intrinsic pontine glioma: a report from the Pediatric Brain Tumor Consortium. Neuro Oncol. 18(5):725-34, 2016
                                                8. Tisnado J et al: Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 31(12):1386-93, 2016
                                                9. Buczkowicz P et al: Pathology, molecular genetics, and epigenetics of diffuse intrinsic pontine glioma. Front Oncol. 5:147, 2015
                                                10. Jansen MH et al: Survival prediction model of children with diffuse intrinsic pontine glioma based on clinical and radiological criteria. Neuro Oncol. 17(1):160-6, 2015
                                                11. Lober RM et al: Diffusion-weighted MRI derived apparent diffusion coefficient identifies prognostically distinct subgroups of pediatric diffuse intrinsic pontine glioma. J Neurooncol. 117(1):175-82, 2014
                                                12. Vallero SG et al: Diffuse intrinsic pontine glioma in children and adolescents: a single-center experience. Childs Nerv Syst. 30(6):1061-6, 2014
                                                13. Jackson S et al: Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma. J Neurooncol. 114(3):339-44, 2013
                                                14. Sedlacik J et al: MR imaging assessment of tumor perfusion and 3D segmented volume at baseline, during treatment, and at tumor progression in children with newly diagnosed diffuse intrinsic pontine glioma. AJNR Am J Neuroradiol. 34(7):1450-5, 2013
                                                15. Löbel U et al: Quantitative diffusion-weighted and dynamic susceptibility-weighted contrast-enhanced perfusion MR imaging analysis of T2 hypointense lesion components in pediatric diffuse intrinsic pontine glioma. AJNR Am J Neuroradiol. 32(2):315-22, 2011
                                                16. Sethi R et al: Prospective neuraxis MRI surveillance reveals a high risk of leptomeningeal dissemination in diffuse intrinsic pontine glioma. J Neurooncol. 102(1):121-7, 2011
                                                17. Steffen-Smith EA et al: Proton magnetic resonance spectroscopy predicts survival in children with diffuse intrinsic pontine glioma. J Neurooncol. 105(2):365-73, 2011
                                                18. Yamasaki F et al: Magnetic resonance spectroscopic detection of lactate is predictive of a poor prognosis in patients with diffuse intrinsic pontine glioma. Neuro Oncol. 13(7):791-801, 2011
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Pathology

                                                        • Clinical Issues

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Abbreviations

                                                              • Diffuse intrinsic pontine glioma (DIPG)
                                                            • Synonyms

                                                              • Pontine glioma
                                                            • Definitions

                                                              • Imaging diagnosis that includes multiple histologic & molecular glial tumor subtypes
                                                              • Glial tumor centered in pons (WHO grades II-IV)
                                                                • Expansile, often poorly marginated, typically lacks enhancement
                                                                • Glioblastoma shows focal enhancement & central necrosis

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          • Image Interpretation Pearls

                                                                                            • Reporting Tips

                                                                                              Selected References

                                                                                              1. Pfaff E et al: Brainstem biopsy in pediatric diffuse intrinsic pontine glioma in the era of precision medicine: the INFORM study experience. Eur J Cancer. 114:27-35, 2019
                                                                                              2. Harward S et al: T2-weighted images are superior to other MR image types for the determination of diffuse intrinsic pontine glioma intratumoral heterogeneity. Childs Nerv Syst. 34(3):449-55, 2018
                                                                                              3. Hoffman LM et al: Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): a collaborative report from the International and European Society for Pediatric Oncology DIPG Registries. J Clin Oncol. 36(19):1963-72, 2018
                                                                                              4. Johung TB et al: Diffuse intrinsic pontine glioma: new pathophysiological insights and emerging therapeutic targets. Curr Neuropharmacol. 15(1):88-97, 2017
                                                                                              5. Mackay A et al: Integrated molecular meta-analysis of 1,000 pediatric high-grade and diffuse intrinsic pontine glioma. Cancer Cell. 32(4):520-37.e5, 2017
                                                                                              6. Hoffman LM et al: Spatial genomic heterogeneity in diffuse intrinsic pontine and midline high-grade glioma: implications for diagnostic biopsy and targeted therapeutics. Acta Neuropathol Commun. 4:1, 2016
                                                                                              7. Poussaint TY et al: Apparent diffusion coefficient histogram metrics correlate with survival in diffuse intrinsic pontine glioma: a report from the Pediatric Brain Tumor Consortium. Neuro Oncol. 18(5):725-34, 2016
                                                                                              8. Tisnado J et al: Conventional and advanced imaging of diffuse intrinsic pontine glioma. J Child Neurol. 31(12):1386-93, 2016
                                                                                              9. Buczkowicz P et al: Pathology, molecular genetics, and epigenetics of diffuse intrinsic pontine glioma. Front Oncol. 5:147, 2015
                                                                                              10. Jansen MH et al: Survival prediction model of children with diffuse intrinsic pontine glioma based on clinical and radiological criteria. Neuro Oncol. 17(1):160-6, 2015
                                                                                              11. Lober RM et al: Diffusion-weighted MRI derived apparent diffusion coefficient identifies prognostically distinct subgroups of pediatric diffuse intrinsic pontine glioma. J Neurooncol. 117(1):175-82, 2014
                                                                                              12. Vallero SG et al: Diffuse intrinsic pontine glioma in children and adolescents: a single-center experience. Childs Nerv Syst. 30(6):1061-6, 2014
                                                                                              13. Jackson S et al: Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma. J Neurooncol. 114(3):339-44, 2013
                                                                                              14. Sedlacik J et al: MR imaging assessment of tumor perfusion and 3D segmented volume at baseline, during treatment, and at tumor progression in children with newly diagnosed diffuse intrinsic pontine glioma. AJNR Am J Neuroradiol. 34(7):1450-5, 2013
                                                                                              15. Löbel U et al: Quantitative diffusion-weighted and dynamic susceptibility-weighted contrast-enhanced perfusion MR imaging analysis of T2 hypointense lesion components in pediatric diffuse intrinsic pontine glioma. AJNR Am J Neuroradiol. 32(2):315-22, 2011
                                                                                              16. Sethi R et al: Prospective neuraxis MRI surveillance reveals a high risk of leptomeningeal dissemination in diffuse intrinsic pontine glioma. J Neurooncol. 102(1):121-7, 2011
                                                                                              17. Steffen-Smith EA et al: Proton magnetic resonance spectroscopy predicts survival in children with diffuse intrinsic pontine glioma. J Neurooncol. 105(2):365-73, 2011
                                                                                              18. Yamasaki F et al: Magnetic resonance spectroscopic detection of lactate is predictive of a poor prognosis in patients with diffuse intrinsic pontine glioma. Neuro Oncol. 13(7):791-801, 2011